Friday, 31 October 2014

PHighter Friday: Kellie

Life beholds many unforgettable experiences.  One of those cherished moments occurred for me on February 1, 2007 when Paul completely surprised me with his marriage proposal.  I was ecstatic to say the least, and we quickly set to work on planning our August wedding.  In mid-February the wedding planning came to a brief halt as I dealt with a short bout of the flu.  Once I was feeling better I dove back into wedding planning mode! However, something was not right as I became more active.  I was unusually short of breath during my workouts.  After a few weeks of trying to "get back into shape" I decided it was time to schedule a doctor visit.  The results came back fine, so once again I decided to start the workouts and push even harder.  By May my breathing was continuing to get worse.  Paul convinced me to set up another doctor appointment.  By the time I set up this appointment I could barely walk from my car into work without having to stop and rest.  At this second visit my doctor decided it was time to look at my heart.  A few tests were done and an echocardiogram was scheduled for a couple days later. 

On Friday, May 25, 2007 I arrived for my echo a bit apprehensive, but hoping I would receive a simple explanation for my shortness of breath.  It wasn't long before I sensed something "wasn't right" during the echo, but the technician wasn't about to reveal what it was to me.  I will never forget the look on my doctor's face when he came in to break the news to me that my pulmonary pressures were extremely high, there was major concern about my heart, they were going to do a few more tests, and then I would probably have to be admitted to the hospital that day (which I was). 

After being admitted to the hospital the panic really set in.  As I was being wheeled away for a right-heart catheterization, I remember looking into Paul's eyes and both of our eyes immediately pooled with tears...tears of fear and the unknown. After the catheterization I was sick, chilled and scared.  Whatever I was given at that point knocked me out for the night.  The next eight days I slowly learned more about my disease and the aggressive treatment regime I would be starting to try halt this progressive illness.  It was decided that triple-therapy was in my best interest, so I began sub-q Remodulin, Tracleer and Revatio.

I spent the next three months focusing on healing and learning how to manage my medications so I would be ready to walk down the aisle.  It was a summer filled with much pain and suffering, but I was determined to move on and live life to the fullest.  By the grace of God, I had the most fabulous wedding day.  On August 11, 2007 I was able to marry my best friend and companion for life.  The challenges of the previous three months had only brought us closer together and made me all the more sure that God was taking care of me in a special way through the gift of my husband. 

One of the most difficult realities of my illness was the loss of being able to bear children. Although my heart ached, I was blessed with an adorable nephew and a beautiful niece, as well as a classroom full of first grade students to enrich my life. I was thankful to be alive and thankful to be back working full-time with children. I was gifted a notepad with the saying, “keep a dream in your pocket and faith in your heart.” This immediately became my motto, as it deeply resonated with the way I wanted to live my life. So I kept on dreaming and had faith in God’s plans for my life. I trusted that my life would continue to be filled with blessings, many greater than I could imagine.

In 2011, my husband and I felt fortunate with my improved and stable health condition and felt it was time to pursue adoption. Just as we finished the paperwork to be listed as a “waiting family,” PH was brought to the forefront in our lives when my mother was unexpectedly diagnosed with PH. That’s a whole other story in itself, but it was a magnificent reminder that anything is possible with God. We witnessed a true miracle in my mother’s recovery and are blessed to have her here with us today.

In 2012 the greatest blessing of our lives came into the world. My heart sings that we were chosen to be the parents of a beautiful baby girl. I will never forget the day she was born and the love that swelled inside of me as I took my first look at her. God had a plan, and it was even better than I could have imagined. My heart that once ached with loss is now bursting with love and I feel at peace. As I watch her play, learn and grow, I am reminded everyday how beautiful and precious life truly is. I am blessed to be a mom.

Since my diagnosis, I was determined to thrive, not just survive. I have taken an active role in doing my best to lead a healthy lifestyle that has allowed me to thrive. I immediately began a low-sodium diet. It was a difficult change in the beginning, but I could sure tell a difference. Prior to my diagnosis I was active in sports that my body no longer tolerates. I have since found new activities that I enjoy and are a benefit to my health. I enjoy yoga and mediation. I love going for walks and recently got a Fitbit so I can track my daily steps and activity. I have also delved into the world of Young Living Essential Oils and am so excited to have something that helps me as I manage the side effects of all my medications and has given me a boost in my overall feeling of wellness. 

I am thrilled to be involved in the study with the implantable pump for Remodulin. It is such a delight to swim and shower without worrying about an external line and pump. It’s amazing to think of all the advancements in therapy that have come about since my diagnosis 7 years ago and I trust that even better things are coming.

I am thankful for the continued support of wonderful family, friends and medical professionals. I am grateful for the Pulmonary Hypertension Association and all that has been accomplished thus far. I will continue to PHight and give my support until a cure is found!

Although the path can be bumpy at times, I am confident that God is with me every step of the way.  I am keeping a dream in my pocket and faith in my heart because anything is possible with God!

Friday, 24 October 2014

PHighter Friday: Donna

 I had my son in April 1988. He was 4 weeks premature because I had pre eclampsia. He was perfect though. I was very lucky. When he was 3 months old I traveled with my parents to Hawaii to visit my brother and his baby girl. She was born 2 weeks exactly after my son. We were there for 2 weeks.  One day while we were there, my brother, his wife, and I decided to climb Diamond Head.  We were climbing and my sister in law was just bouncing up the volcano.  I was having a rough time and said, well, I can't blame it on just having a baby because she just had one too. Then we finally got to the top and there were about 30 stairs straight up and I looked up and said. What's wrong. I can't do this. But my brother was very patient and I finally got to the top and the view was GREAT.

Came back home, very difficult marriage.  Very difficult. I got sick and couldn't shake the cough so I was sent to a pulmonologist, he gave me inhalers to treat me for asthma.  That didn't work, 2 weeks later I went back and he added more inhalers and did a chest X-ray. He remarked about this big thing on top of my heart but he didn't know what it was. Took the X-ray to the radiologist at the local hospital. That dr said ah that's normal.

Later that year, I was going through a divorce and the doctors I worked for ( I was a nurse), thought my racing heart was from stress so they gave me something for stress. That didn't help my heart slow down so they thought I had Mitral valve prolapse. They gave me a Beta blocker. This was in November 1991. I started the Beta blockers, now no one checked me for anything. I passed out one day at work so they sent me for an echocardiogram.  That didn't show mitral valve prolapse so they sent me the next morning to a cardiologist. I was put on a treadmill and within 3 minutes my heart was racing to 180. They pulled me off immediately. The sweet doctor said "I can't be positive but I think I know what is wrong with you, but you need a right heart cath." Okay this is getting scary but I was young, healthy, free of my horrible ex husband, there couldn't be anything bad.  A couple days later I had my heart catherization, my mom and pastor was there. Well, the weirdest thing happened- my mom acted as if she had been crying and said she was going home to take care of my 3 year old, my dad had him.  Weird, because my mom had never left me in the hospital alone. That evening my best friend came and brought me a beautiful long, lacey, cotton night gown.  So sweet- but unnecessary, I was fine. Then my brother dropped by with a hairbrush, magazines, and chocolate.  That should have sent off warnings but it didn't. Ignorance is bliss at times

The next morning the nurses came and took me for a pulmonary function test. When I got back to my room, my mom was there and my doctor. Mom had tears in her eyes and Dr. Fonenot said down on my beside and said " you have primary pulmonary hypertension ". I said "okay, how do we fix it?"  He said "we don't." I said "okay." He said "it's not okay, you will die in the next 2 years without a heart lung transplant." I laughed and pulled the covers over my head. I looked out from the covers at my mom and she was crying, my doctor was crying.  Okay- this is real.  All I wanted to do was go home and hold my son tight.

The doctor called the next day and said he had found a center that would do my heart/lung transplant in Birmingham Alabama. Wow. That close? About a week after my diagnosis it hit me, I was dying and soon. I cried a couple hours, got down on my knees and asked God to take care of my baby boy.  We scheduled the appointment with UAB in Birmingham for December and  my mom, dad, baby boy and I went to meet Dr. Bourge, the transplant doctor.  He agreed with my local cardiologist and scheduled me for a transplant evaluation for the first full week of January 1992

January came and all arrangements were made for my hospital stay that would take a week. The transplant team had made mom reservations at a hotel close to the hospital and even made arrangements for the hospital day care to keep my son during the day so mom could be with me.  We arrived on Sunday and found the daycare so mom wouldn't have any trouble finding it the next morning.  We found it easily enough but pulled up to the door and they had a sign on the door that they had chicken pox.  We had no choice, we had to leave him there and get exposed.  I was admitted and went through so many test, was told I needed to loose about 10 pounds because I needed to be the size of a 12 year old boy. The tests were never hard but always invasive. They drew around 20 tubes of blood a day. More radioactive tests than I can remember. My veins were so sore and I was so tired and got sick from all the dyes they used. Then on Thursday, the chaplain came in to see me, I had already seen psychiatrists, but they brought in the chaplain, he was one of my old pastors.  I felt so blessed and that I was doing the right thing. On Friday morning I had another right heart catheterization  and the most wonderful thing happened. My pressures were half of what they had been in November.  The Procardia was working. Yay!  The next day we came home with a plan. Low fat, low sodium diet and walking daily. I stopped dying and started living. I dropped the 10 pounds then 10 more. I looked and felt great.

My parents and my son and I spent a week at the beach just tanning, playing, fishing. Enjoying each other and life.  I was working again as a nurse and doing great. The death sentence hung over us but I wouldn't let it dominate me. The next weekend after coming home from the beach, a really good friend asked if I wanted to go out dancing on Saturday night, sounded good.  Mom and dad were watching my son. Well, I was coming out of her hall and this man walked in the front door and we just stared at each other.  He was a cowboy, I was a tiny woman with a big smile. We both will tell you- it was honestly love at first sight.

We started dating immediately and he was wonderful with my son. We had been going out a couple of months and my mom told me that I needed to tell him about my PPH (pah), so one night we stayed up all night and I finally got up the nerve to tell him I was dying and he held me and said " I already know, Ruthie told me".  My friend. Haha well we dated a while, I had a right heart catherization every 3 months.  Since there were no medications for pulmonary hypertension we had to keep a close eye on my pressures. 3 days before Christmas, Milton, my cowboy, took my son to see a movie in Mobile.  That night after I got off work, Milton asked me to marry him. I asked if he was sure because at the time we thought I only had 1 year left to live.  He said he wanted to marry me even if he only had 1 week left with me. We were married in June 1993.

I continued to have heart catherizations every 3 months. In 1995 I had a punctured lung during one of my heart Caths and I spent a week in the hospital with a chest tube. Horrible pain. I was still holding okay on my PA pressures, they stayed in the upper 40's.

Life was good for a long time, except for all those heart Caths. Somewhere along the line I was put on Coumadin and then lasix. Decided I didn't want to find a different doctor. Dr. Bourge and I didn't really get along all that well. I am a people person, he wasn't. Great doctor just not for me. So I was sent to Nashville and saw a wonderful PH group at Vanderbilt.  I had a bubble study there and they were convinced I had a hole in my heart and they could fix my PH. Wow!  After all this time we had hope. They sent me downstairs to a pediatric cardiologist who showed us what he would use to repair the hole in my heart. So the next morning I had an 8 hour long heart cath looking for a hole in my heart. After all that we were back to my original diagnosis of idiopathic pulmonary hypertension.  I couldn't keep going to Nashville, it was just too far away. So I found a doctor in New Orleans. The closest one yet.  I liked him and I had started getting worse. Luckily Tracleer had just come out so he added that to my calcium channel blocker.  That started working and I was doing better. Then he got an offer he couldn't refuse from Houston so he moved and I was without a doctor. I asked his nurse about someone and she told me about a Dr. DeBoisblanc. Thank goodness, he goes by Dr. Ben. We met and it was a perfect doctor/patient match. He was all about my quality of life like I was.

I started getting sicker again and he had heard about trials of Viagra, so we talked my insurance company into getting me Viagra. They sent me 3 months worth of that golden medication. Haha joke. I started taking it and the headaches were horrible, they just got worse and I started vomiting daily. Talked to Dr Ben and he said to stop taking it.  He was trying to keep me off IV medicines. Then sub-q Remouldin came out and he said. You'll love this. No IV. So my local cardiologist worked with him to get me the medicine. I went in the hospital to get started and trained on it. Everything was good for 24 hours and I got to go home. At the 25 th hour the site pain started. Oh no this is awful. On the 3rd day it got better, then I had to move it and titrate up my dosage.  Here we we again. The worst pain ever.  Then it would get better, then I'd have to start over again. It got so bad I started vomiting all the time again. I only got out of bed to vomit. It was awful. So 4 months of that and Dr Ben looked at me and told me to stop that medicine. He said this is not quality of life. Well then, Ventavis came out. Big bulky pro-dose machine that had to be plugged it. Every 2 hours I had to inhale the Ventavis   No side effects and between Ventavis, Tracleer and Norvasc I was doing great. Then we took a direct hit from Hurricane Ivan and were without power for a week. We had to use an inverter in the car so I could do my Ventavis every 2 hours.  Finally the little portable inhaler for Ventavis came out and I was one of the first people to get it. That made life so much easier.  I stayed on these medications until 2007. I started getting worse again. So as soon as Letairis came out I switched from Tracleer to Letairis .  The Tyvaso came out and my local cardiologist told me it was only 4 times a day.  So I changed from Ventavis because I felt like it would be easier for me. I responded really well to Tyvaso and loved how easy it was to me. I have beed on Tyvaso, Letairis, and Norvasc since 2007.

This is the year 2014 and in November that will be 23 years since I was given 2 years to live. I still have my original organs and hope I always will. I have still avoided IV medications but now there are 3 different ones available if the need arises

I still live my life to the fullest. I get tired more but I am now 53. So is it my PH or is it my age?  Who cares. I am going to live to be an old lady. My son is 26, I've been married to my cowboy for 21 years. I have cows, horses, goats, chickens, a cat and a wonderful Boston Terrier that is my little boy. Haha

*Editor's note: you might recognize Donna's story from my previous post where I posted this video of Donna who shared her PH journey with a local news station (video below.)

Friday, 17 October 2014

PHighter Friday: Jeannette

I was 22 when I graduated from college in 1974 and had signed my first teaching contract at a K-12 school district in Thomaston, ME.  I was teaching physical education, health and also coached four sports during the school year. I absolutely loved what I was doing and had a wonderful first year teaching experience. As the next school year started that was about to change drastically! During basketball tryouts in November, while running laps with my girls, I fainted!

After going from doctor to doctor searching for answers, I was sent to a cardiologist at Eastern Maine Medical Center. On May 5, 1976 I began my “real” race with pulmonary hypertension and essentially, over the next 22 years, EMMC would become my second home. May 5th was the day I had my first heart cath.

I was extremely lucky to have been sent to a cardiologist who knew I needed a heart cath to determine I had PH, and I am truly thankful to him for that.  The news he gave me was very disheartening:  “You have, maybe, two years to live. The time you have left will be very gloomy.” He also said to me, “There is no treatment and there is nothing else I can do for you”. And, fortunately for me, he left the practice. Who would be able to help me? Dr. Joe Wise took over my case. He told me as long as I wanted to keep trying, he would do everything he could to help me survive.

Shortly after our engagement, David and I learned of my prognosis. I doubted myself as a woman and future wife; my whole world had been turned upside down.  I wasn’t dealing very well with my situation. How was I going to adapt to becoming David’s wife? How could I handle a marriage when I couldn’t even handle myself? Was this fair to him?  David never considered not marrying me. This tells a lot about the person he is and has been during this whole journey. It has not been just my journey; it was and is our journey.

June 25, 1976 was our beautiful evening wedding. We spent the summer in my home town, in a camper on a piece of my brother’s land overlooking a beautiful stream, in the woods. I spent a lot of time sitting by a campfire, pondering, writing and trying to figure out what I should do with the time I had left. I did a lot of reading and Kubler-Ross’s book entitled “Death and Dying” was very helpful in my efforts to understand and deal with the prognosis I had been given. The realms of emotions from day to day were so varied; anger, sadness, relief to know that this had not all been in my head, confusion, withdrawal; wanting to talk but just not able to; was this really happening to me?  I was good at shutting people out but not very good about letting them in.

One week after I was married, I was back in the hospital for a tubal ligation, as I would never have been able to survive a pregnancy. This was very difficult because I had always planned and looked forward to the time that I would be a Mom. David and I would not be able to have children. It was devastating for both of us.

 “How could I have gotten a disease that, at that time, only 1 in 2 million people had?” I was able to find one article about PH. It was about a half page long, echoed what I had been told, with the exception that once fainting begins, the prognosis drops to 6 months. I had been fainting with very little exertion since November.

I blindly went back to Thomaston to start the new school year. I was going through the motions of living; hiding from the reality of my situation. November arrived and I hit rock bottom. I took an overdose of my heart medication. I was nervous, scared, upset and embarrassed, but at the same time I remember thinking: maybe I can get some help to deal with this. Even during this devastating time I had some hope. I was overwhelmed and did not know how to move ahead. Everything was all mixed up like a big ball of yarn. I couldn’t find the beginning, and the end just looked like darkness. I was afraid! You are 24 years old and you are dying!

One of the toughest times of this journey was the day I was admitted to the Psychiatric floor at EMMC. Clinically, I was in denial about dying and depressed about my limitations. I learned a lot in my two week stay including some skills for coping. It wasn’t my fault that I got this disease. I hadn’t been a bad person. I didn’t have to blame anyone. This was the little flicker of light in the darkness I had been looking for. I was able to write a letter to the disease, put it in a box, and place it on a shelf along with the anger. I chose to accept it and leave it abandoned. What a difference that made! A weight had been lifted. I was ready and able to move ahead, and felt confident and better equipped to handle my life.

It did not take long for me to figure out that if I was going to remain mobile, I would need some assistance. My physical limitations had become very apparent. Walk a few feet, lie down, try to relax enough to prevent fainting, and then try again. Dr. Wise suggested an electric wheelchair so that I would be able to be outside, go to events, and go for walks. The only stipulation was I would continue to exercise as best I could. After a bit of hesitation, I decided to swallow my pride of having to ride in a wheelchair and gave it a try. After the first few times, I became comfortable. I was able to go camping and get around independently.

In April 1979, I took an expected but undesirable turn for the worse. David and I thought this would be my last stay in any hospital, as I had started to faint while lying down, so we were preparing ourselves. Dr. Wise had just returned from a medical meeting, learned that a new antihypertension drug (Nifedipine) had shown to reduce PA pressure in some patients, and a new catheter had been developed which could measure pulmonary artery pressure at the bedside. I was started on it and improvement was amazing. I went from not being able to walk more than a few steps to walking the length of the hospital hallway. I returned home and started training; walking the distance between telephone poles, first one set, then two sets until within a couple of weeks I was walking a mile. I was able to return to teaching in 1981. Teaching physical education, however, proved too much for me, so I became certified to teach elementary education. I found the classroom perfect. I was doing what I loved; teaching and coaching.  I was doing so well, that, after talking with Dr. Wise, David and I decided to pursue adoption. On April 30th, 1987 our two sons were home. Truly a miracle is happening when you start an application in June for an overseas adoption and ten months later you are a family of four.

Dr. Wise moved to New Mexico in 1987, and I must say that was a tough good-bye. I started seeing Dr. Silver, who I knew very well and who had the same philosophy as Dr. Wise about my care.

In the fall of 1996, I had pneumonia, which required hospitalization. I recovered, went home and work, and in January I got it again and recovered. After several visits and tests Dr. Silver knew that nifedipine no longer had the effect it had prior to the pneumonias. He told me he had been in touch with Dr. Robyn Barst at Columbia-Presbyterian Hospital in New York. He was concerned about my increasing PH symptoms. In March, 1997, I had a consult with Dr. Barst. There were many tests during this consult. I met, for the first time, another PH patient, 21 years after my diagnosis.  Someone knew how I felt without trying to explain. I didn’t realize how isolated I had been until after talking with other patients.  I learned about PHA and support groups. Even though the nearest support group was almost 3 hours away from my home, I would start attending as soon as possible.  The toughest news of this visit was learning I would have to retire from teaching, for a second time.  I did not want to have to go through this again but knew I had no choice.

In April, I was admitted to Columbia-Presbyterian. David, Ian and I learned how to mix flolan and all the “what to do and how to do it.” I had the catheter placed and my life with flolan began. I was nervous leaving the hospital, but I knew it was time to return home. It was a bit scary that only the three of us knew anything about how to mix flolan and all the other intricacies of using it. I had regular visits in New York, twice a year. I was increasing flolan on a regular basis and doing quite well.
March, 1999 found me having lung surgery for a suspicious nodule in my lung. It was a relief to know the nodule was not a problem.

In early March, 2000, I started having problems with shortness of breath and fatigue. My life was: get up, wash up, return to bed, read the paper, fall asleep; get up again, take care of the catheter site, mix my medication and then lie in my recliner. I couldn’t eat without vomiting and stopped eating. My source of nourishment was strawberry/kiwi Gatorade.  By late March, I could not hold that down. I had lost 50 pounds and my ph symptoms were intense. My primary care doctor was very concerned and wanted me to see a specialist as soon as possible.

David came home from school at noon on Friday, April 12, searched the phone book, called Maine Medical Center and asked if anyone there treated “Primary Pulmonary Hypertension”. He was told to call Chest Medicine Associates. Karen, the PH nurse, arranged for me to be admitted to MMC that day, to the Special Care Unit, and the following morning I met Dr. Joel Wirth.  There were a number of issues going on and my hospital stay lasted three weeks. Rheumatoid Arthritis and Thyroid disease were added to the health record.

September 23, 2003 found me at Maine Medical Center for surgery to have my thyroid removed. I received the news that the thyroid was cancerous, but the surgeon was certain he got it all.

At 7:30 a.m., April 1, 2005, I became bald and had surgery, yet again, to have a shunt placed in my brain for a condition called pseudotumor cerebri. Not a very nice April Fool’s Day!

I had attended my first PHA International Conference in Miami in 2004. I learned so much, and met many other patients at conference. I wanted David to experience conference too. We have not missed a conference since 2006. At the Minneapolis Conference, we heard Mr. Carl Hicks talk about fear; how fear prevents us from fighting, especially for a cure for PH. This had a profound impact on me and I took his message to heart. I needed to step up and become involved.

We wanted to raise awareness and funds for pulmonary hypertension. I have a passion for golf and so we decided, “Why not a golf tournament?” In 2007 the first annual Maine “Swinging for a Cure” Golf Tournament and Auction was held. 

In September, 2006, I transitioned to IV Remodulin. My current PH therapy is: IV Remodulin, Revatio and Nifedipine.

In July, 2007 David and I took a trip we never thought we’d ever be able to make. Our sons, Brian and Ian, had returned to visit the country of their birth. We wanted to experience the Korean culture and see the boys’ homeland as a family. It took some careful planning on our part and we did it!
My book was published in June, 2010.  Dr. Wise, right after I became his patient, encouraged me to journal. He thought this would be a good way for me to express “my hidden, unsaid” thoughts. After finding PHA, support groups, and becoming more involved with other patients, I started putting the journal writings into book form. Many encouraged me to put it in print. It was not easy to write all the ups and downs and the very lowest points along the way, but I tried to be honest about the challenges and how I got through them. The ultimate goal for this book is to give others with PH hope.
I received a phone call from Meryle Reeesman in April, 2012, telling me that I had been chosen as PHA’s Outstanding PH Citizen. “Are you serious?” I asked. I didn’t realize how I would react when I stood on the stage and received the award. I could not stop the tears as I looked out over the banquet room which was filled with over 1500 people. Flash backs of the past 36 years all flooded in around me. I have always carried hope that a cure would be found, and I truly believe that a cure will be found.  In 1976, I never dared to imagine seeing so many people all in one place, coming together, talking, sharing research papers, and collaborating about PH. It truly was overwhelming for me.

In September, 2012:  I started to have leg pain in my upper right thigh. X-rays didn’t show anything. By January I was using a cane, then crutches. On April 8, my rheumatologist told me I had a hairline fracture in the femur. I found myself in surgery on the 10th having a rod placed in the femur. The surgeon told me that it was most likely caused from the drug fosomax – only 4% of patients who use fosomax get this condition. I did fine through the surgery. I returned home on April 15th. The recovery at home took several weeks, but I was able to recover.
I never thought I would ever be able to say this: “I am a Grammy”! How awesome is that! David and I traveled again to South Korea in June, 2013 to visit our new grandson, Kai. Oh, what a special trip that was! Holding my new little grandson was just one of the most precious, special moments of my life.

There was no way I was going to miss Kai’s first Christmas; even if it meant another flight to South Korea, so on December 13th, Ian, David and I arrived in Incheon. It was just a wonderful first family Christmas! We returned home on December 31. And then…
It was back to Portland and a visit with the surgeon who had done the surgery on my right leg. Two days before returning home, my left leg gave out on me. Same pain as before; I knew what lie ahead. On Jan. 8, I was back in the hospital to have a rod placed in my left femur. Again I did fine during the surgery but the recovery was very difficult. I returned home 5 days after surgery. I was using oxygen 24-7, which is not what I am used to. On February 4, I woke up, sat on the edge of my bed, still not doing well, tears running down my cheeks and asked myself, “Am I going to get through this? Yes, you are!” I had to work very hard to discontinue the oxygen and get back up on my feet. It took two and a half months to be up and moving again.

Another road block; at my routine ophthalmologist’s appointment, I learned that I needed to have cataract surgery. I had both eyes done and I did very well. On June 2, I was able to swing my club and play 9 holes of golf.  It was a great day!

Becoming a member of PHA has allowed me to connect with many people all over the world. I have been a support group leader in Maine since 2004.  I have had the opportunity to speak at PHA’s Congressional Luncheon on two occasions in support of the PH Research Act. Being a member of PHA is a good way to include your family members in your journey with PH.  There is so much to learn. Conferences allow you to gain a much better understanding of PH; armed with updated information to take home and share with others. My attendance at conferences has given me a sense of empowerment over PH; but the most important thing gained from attending conference is:  You discover that you are not alone!

I have shared with you the many obstacles I have faced since my diagnosis to the present which required me to really think about what I needed to do to stay positive and live. I would never say that it was easy; it wasn’t and it isn’t; but there is the other side. My life is not what I thought, or expected it to be. For me to have experienced all have experienced is amazing. The early days, months, and years were very difficult and yet; at the same time made me aware of what was truly important. The only way I can explain getting through them was that I know I had someone walking with me and watching over me.  My faith has grown and I know it has gotten me to this point in my life.  There is a reason and purpose to everything that happens in our lives. Right after my diagnosis, I told my older brother about having “pulmonary hypertension” and ending the conversation with “it is not going to take my life or anything like that”. I know I didn’t believe that. The statement came from deep within. And the years keep moving along. When I turned 30; I thought that was a miracle. I turned 62 in August! I call that a precious gift. 

The one thing I feared most after diagnosis was that I would not be able to accomplish anything in the time span given me. What was I going to be able to do in maybe two years that would be beneficial to anyone? I did not want to look back over the time I had left and have to kick myself in the butt for not doing anything. I think this was a great motivator for me to set a goal of doing something, anything each day. I learned to live with Pulmonary Hypertension, accepting and adjusting, but still trying to live a productive life, not knowing the outcome.
I asked Dr.Wise if he would share some of his thoughts about my case so I could include them in my book. An excerpt of what he wrote follows: “When I met Jeannette she was 23 years old. She had what everyone agreed was terminal right heart failure, the result of severe primary pulmonary hypertension. She required hospitalization on a regular basis for removal of edema fluid – symptomatic treatment at best – for, in spite of extensive consultation, there was no known treatment for the underlying condition. Between hospitalizations she was essentially bedfast. I learned two important things from her. One, it is very difficult to know, with any confidence, how long someone might live or whether a disease is too far advanced for cure. And two, it is very nearly impossible to extinguish strong hope.”

My early years of isolation, loneliness, no one really understands my disease, to hear, “You look so good”, when you are feeling so poorly; is tough to deal with alone. You and I don’t have to; there are so many who have the desire to help. I encourage you to reach out and find the support that will help you stay strong as you continue on your journey.

I learned something a few years ago from Mr. Carl Hicks, whom I love, respect and admire, when I heard him say, “Hope is a verb”. We cannot let fear paralyze us, or remove hope on our individual journey.

Thank you for allowing me to share my story! Please feel free to contact me at

Friday, 10 October 2014

PHighter Friday: David

Where to begin…hard to remember life before PH day.

I was always an active person, loved running, loved football and took the ability to surpass other people’s fitness for granted.

I guess I had it all, great kids and wife a baby only just on the way, great job and prospects running the operating theatres in Preston England, and an active lifestyle.

Then after a lay off after a sporting injury I sated to try and run again. And I couldn’t. Literally couldn’t run 50 meters without feeling like my chest was caving in.

I passed this off as being unfit and enjoying too many pizzas and beer, but when it was commented on by a member of my team on how unfit I was after walking up stairs and being breathless to the point I couldn’t talk, something triggered in my head and I went to see my GP. I thought it was Asthma or Hay fever-but was surprised to be referred to cardiology for Echocardiograms and CTs-but still I wasn’t really bothered-indestructible.

Then I got the call-URGENT CT and my knees buckled a bit. Still went in with good humour-until I got the results-my consultant sat down me down with my wife and proceeded to talk about how my heart was enlarged due to significant pressures in my lungs and all went hazy. I can’t remember before that conversation now-1830 on the 22/8/13 In Chorley Hospital.

Although I am medically experienced, I had never heard of Pulmonary Hypertension and there was little information in the hospital I was in, so I did what any 21st century boy would do, and I asked DR Google and that’s when it all went black.

All I could think was ‘I’m going to die and leave my wife and kids without a dad and husband’, I had never been so scared and  unpowered in my life- I was a manager of people-I dictated the rules-not this time

Then it suddenly became worse-

‘David-you need to have HIV tests due to your age, you have Tattoos and this is an indicator for HIV’ I couldn’t breathe, it is hard enough to worry about leaving family, but to leave them and also condemn them to that crippled me. I couldn’t talk for the week that I waited for the all clear.

This was by far the worst experience of my life.

To be fair the hospital care over the next 2 weeks was good-I was looked after by a great team both in Preston where I was transferred to and then to Sheffield (which is my specialist centre). I was told I had idiopathic PAH although I can directly link onset to running a Half marathon then flying to Australia a couple of days after-I was never the same after that.

I experienced many procedures which previously as a theatre practitioner had imposed on other patients, and came out of it a better practitioner.

Following discharge started life again, having to get used to boxes of drugs to organize a month and become regimented and there I dwelled occasionally going to clinics where I would be sat in-between patients 3x my age. I was too scared to move, for months I counted and limited every step and breath as if it was my last and let myself be overcome by a black shadow, I arranged life insurance, redirected my pension thinking everyday was my last, not wanting to spend money on haircuts etc. as what was the point. Immersed myself in my PS3 and just sat for what seemed like months. I couldn’t sleep, I’d dream that I was having cardiac episodes or dying so vivid that I was screaming in my sleep- I really feel bad for how little I spoke to my wife and kids although I do know that I was battling depression.

In addition to this I was having chest pains and palpitations which screwed my nights up. I’d just sit every night watching Predator over and over. It later turned out to be reflux which yet another tablet would resolve-but goes to show-not every bump and ache would be due to my heart.

From that point on I slowly picked myself up-started to go back to work, first 2 days a week, then 3, then 4.

At each stage I was feeling exhausted and worried that I couldn’t go back to my old job which was stressful, demanded long hours and fast paced, but the hours worked started to get longer and I was getting more comfortable racing from meeting to meeting, but it was still at the back of my mind, the shadow that constantly required me to count my steps-to be worried at every flight of stairs I encountered-just in case I couldn’t climb them and id be reminded and I’d start from the beginning again.

My real turning point came at my 9 month appointment at Sheffield. The 6 minute shuttle run. On diagnosis I could only make 800m-that haunted me. This time I focused and I nailed it, I could have done more-I almost cried as I had beaten my demon (but always in my mind a voice said ‘why are you happy? You have only jogged 1000m you were running half marathons)

The next steps were to start going to the gym again before each step I was so nervous-this could set me back-I don’t want to be reminded that I was limited, but I didn’t want not to know either. The only way I’d be happy was to confront it head on-I was back, feeling mentally  and physically strong and fighting the entire time-IPAH pushed to the back of my head-always trying to escape but it push it back again.-id wake up knowing I was fighting and went to sleep ready to recharge.

I started off slow and for 10 minutes-I limited myself-I could have done more but was too scared. Then one of the most influential men post IPAH challenged me. MR Saba from Blackpool hospital said-we’ll never know how bad it is unless you push it. Game on!! running increased to 30-40 mins-granted it was half the speed that I was used to but I could run-without breathlessness and other associated symptoms, and gradually the speed has risen, not by a lot but it has, since then I’ve lost loads of weight and I go to the gym 3 times a week for at least 2 hours at a time. I now do interval training and have pushed myself as hard as I ever had done.

Meeting people on line in forums was a life changer too.

People with my condition alive after 20+ years and not the 2-3 that Dr Google had suggested?

 Also that I was far fitter than many people on the forums-I was working 50 hours a week-intense work, gymming and coming home to 4 kids and not once was I out of breath or symptomatic, I don’t need O2 or may drugs, just 25 sildenafil, and Warfarin.

I don’t take this for granted now. I have seen how this has affected some people physically and mentally and I’m not going to be the nest victim. It has to beat me and I don’t get beat. That’s not to say I’m arrogant-this condition and the days after diagnosis still haunt me and the black cloud still lingers, I occasionally tear up and want to cry, and I know that I’m still hard to get talking and prefer to be out of crowds, but I’ve accepted this challenge-it’s a long one, but a long fight is better than 2-3 years.

I’ve recently put my name down for a charity 5k run for PHA-my next challenge which scares the bejeezers out of me but again, I have to do it, and I’ll run through the fear.

My consultants have called me remarkable and have been discharged to yearly Sheffield appointments, so all in all not the best thing to happen to me, but I’m still alive.

What would I say to the newly diagnosed?

There’s no point saying avoid google, because either you or a loved one will.

Just accept it and move with it. Don’t get caught up in how it affects others; get caught up in how it affects you. Be inspired by these PHighters by all means but one size doesn’t fit all, push yourself past every boundary-no matter how small they may seem to ‘normal people’ or how you would have been before.  Just get out and do it and fight the fear otherwise you’ll be enveloped by the worst thing possible. FEAR.

I couldn’t have done this without my wife and 4 girls. They are my power, I have very little family around me, and although I know it’s been really hard for her to really accept and understand how black it was inside my head and how hopeless life seemed even when amazing events like my daughter Lorien was born, she reminded me that life is never hopeless if you don’t lose hope.

Update: David completed a 5k run in 36 minutes after his blog submission. Way to go, David!